KDIGO 2025 ADPKD guideline: a commentary on diagnosis and management of hepatopancreatic manifestations by the ERA Working Group Genes & Kidney.

The Kidney Disease Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a long-awaited development and the first KDIGO guideline addressing a genetic kidney disease. The approval of tolvaptan as targeted therapy for ADPKD in 2015 marked a pivotal advancement and motivated a structured approach in research and clinical care. The systemic nature of ADPKD guidance for the management of extrarenal manifestations is of special importance for nephrologists and requires interdisciplinary collaboration. In this regard, cysts in other organs, particularly the liver, are the most common extrarenal finding. While most liver cysts are incidental findings on routine imaging, both cyst infections and severe polycystic liver disease accompanying ADPKD come with a high disease burden and challenges in management. In contrast, pancreatic cysts in ADPKD are usually completely asymptomatic, but this raises questions about the need for follow-up. This commentary provides an overview of the most important recommendations and practice points on hepatopancreatic cysts provided by the KDIGO guideline with the aim to contribute to implementation and highlight remaining gaps in guidance, clinical care and research.